A complete case of Cantrell's Pentalogy with isolated left ventricular diverticulum

The congenital left ventricular diverticulum is a rare cardiac malformation, and it may associate with Cantrell’s Pentalogy with other cardiac defects. However, isolated ventricular diverticulum without any other cardiac defect in complete Cantrell’s syndrome is very rare. We describe a 6-year-old male patient with a complete Cantrell’s syndrome with isolated left ventricular diverticulum.

Myocardial Expression of Estrogen Receptor-mRNA Is Associated With Lower Markers of Post-operative Organ Damage in Young Patients With Congenital Cardiac Defect

Background: Estrogen receptors (ERs) relate to cardio-protection in adults, but their role in younger patients is not known. We aimed to assess the myocardial expression of ERα- and ERβ- mRNA in young patients with congenital cardiac disease and to analyze their putative protective role.Patients and Methods: Twenty children and young adults (seven females and 13 males) with a median age of 13.8 years (interquartile range: 12.3 years) were enrolled in this prospective study. The myocardial expression of ER-mRNA and genes involved in inflammation, growth, and stress response was assessed by real-time PCR and was correlated to post-operative (po) outcome.Results: ER-mRNA was detected in the myocardium of all patients, independently of gender and age. The expression of ER-mRNA correlated with that of mRNA coding for brain natriuretic peptide and for all cytokines tested. A higher ERα-mRNA expression correlated with lower troponin T concentrations at 24 h po (p = 0.032), higher PaO2/FiO2 ratio at 4 h po (p = 0.059), lower fluid retention at 4 h po (p = 0.048), and lower aspartate aminotransferase (AST) levels at 24 h po (p = 0.047). A higher ERβ-mRNA expression was also correlated with lower fluid retention at 24 h po (p = 0.048).Patients in whom the levels of ERα- and ERβ-mRNA were >P50 had lower troponin T (p = 0.003, respectively) and lower AST concentrations at 24 h po (p = 0.043, respectively) than the others.Conclusions: The expression of ERα- and ERβ-mRNA is present in the myocardium of children and young adults with congenital cardiac defect and is associated with lower markers of po organ damage. This suggests that ERs may provide perioperative organ protection in this population.

Spinal abscess in a patient with undiagnosed gerbode defect: a case report

Background Gerbode defect is a rare cardiac defect in which an abnormal communication occurs between the left ventricle and right atrium. The aetiology is usually congenital but acquired defects can occur. Case summary We report on a 47-year-old male with atrioventricular block prior to decompression of an epidural abscess extending from the skull base to the seventh thoracic vertebrae. Following positive blood cultures for Staphylococcus Aureus, a transoesophageal echocardiogram performed revealed a small Gerbode defect with associated endocarditis. In our case, the defect was small and there was no evidence of heart failure, there was little guidance or literature available on how to best manage our patient. A multidisciplinary decision was taken to treat the endocarditis medically and to not close the defect in the acute setting. He recovered well and did not suffer any further cardiac complications. A repeat transthoracic echocardiogram did not reveal any evidence of endocarditis. Conclusion Gerbode defects are rare but have been known to increase the risk of developing endocarditis. It is important to have a high clinical suspicion of endocarditis in patients with evidence of conduction disorders and systemic infection.

An extremely rare association of coarctation of aorta with double chambered right ventricle: double-trouble causing bi-ventricular failure in a child

Double chambered right ventricle is a rare cardiac defect characterised by an obstructive hypertrophied muscle bundle in the right ventricle. The common associated lesions are ventricular septal defect followed by sub-aortic membrane. We report a child who had coarctation of aorta in association with double chambered right ventricle. This case is being reported for its rarity and challenges in management.

Evaluation and Management of Pulmonary Arterial Hypertension in Congenital Heart Disease

Pulmonary arterial hypertension is a common complication in patients with congenital heart disease (CHD), aggravating the natural course of the underlying defect. Pulmonary arterial hypertension (PAH) has a multifactorial etiology depending on the size and nature of the cardiac defect as well as environmental factors. Although progress has been made in disease-targeting therapy using pulmonary vasodilators to treat Eisenmenger syndrome, important gaps still exist in the evaluation and management of adult patients with CHD-associated PAH (PAH-CHD) who have systemic-to-pulmonary shunts. The choice of interventional, medical, or both types of therapy is an ongoing dilemma that requires further data. This review focuses on the evaluation and management of PAH-CHD in the contemporary era.

Primary causes of In-Hospital Mortality in Pediatric Cardiac Surgery Population

Objective: To present primary causes of in-hospital mortality in large surgical population who underwent surgical correction for CHDs for the period of a decade at our institute. Methods: Retrospective analysis of pediatric cardiac surgery database along with decision of morbidity and mor-tality meeting at Department of Cardiac Surgery at CPE Institute of Cardiology from January 2009 to April 2021. Results: An overall 3705 patient underwent surgery for CHDs. The in-hospital mortality for CHDs surgery is 2.37% with RV dysfunction, Residual cardiac defect with valve dysfunction (TR/PR), pulmonary hypertensive complication, mediastinal bleeding, cardiac tamponade, respiratory complication, MODs, heart block as major primary cause of in-hospital mortality with frequency of 15.91%, 18.18%, 18.18%,18.18%,9.09%,10.23%, 4.54% and 4.54% respectively. Conclusion: in our population of 3705 congenital cardiac surgery patients, incidence of overall in-hospital mortali-ty is promising for wide range of CHDs. RV dysfunction, residual RV defects with vale regurgitation, pulmonary hypertension, bleeding complications and respiratory failure are major primary causes of in-hospital mortality. Keywords: In-hospital Mortality, Atrial septal defect (ASD), Tetralogy of Fallot (TOF), Ventricular septal defect

Rupture of Aortic Sinus Aneurysm Caused by Trauma

Background: Sinus of Valsalva aneurysm is just an uncommon cardiac anomaly, a congenital or acquired cardiac defect that is present in roughly 0.09% of the general population. With an incidence of less than 1% of opening heart surgery cases.Case Presentation: A sinus of Valsalva aneurysm is just an uncommon cardiac anomaly. The aneurysm usually ruptures into the cardiac cavity. The most frequent complication of SVA is rupture into the right atrium or right ventricle. We reviewed 2 cases of rupturing sinus aortic aneurysm.Conclusion: Echocardiography examination needs to monitor the diameter of the aortic sinus, aortic root and aortic valve, especially in the army.